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Biomedical subjects

R Goldberg

Publications and source records attributed to R Goldberg.

At least 145 records · Page 8Linked to original sources

Effect of dialysate composition on the lipid response to L-carnitine supplementation.

Cumulative carnitine losses through dialysis membranes may worsen hyperlipidemia during long-term hemodialysis. However, carnitine supplementation has not shown a consistent beneficial response in hyperlipidemia. We have compared in a double-blind, cross-over study the effect of dialysate buffer composition (acetate or bicarbonate) on the serum lipid response to L-carnitine supplementation during hemodialysis. We studied nine patients (mean age, 19 years; range, 14 to 23) with hyperlipidemia undergoing maintenance hemodialysis. Plasma levels of carnitines and lipids, including total and HDL cholesterol (HDL-C) and triglycerides (TG), were measured at baseline and monthly intervals after receiving 2 grams of L-carnitine or placebo added to dialysis bath for three months. One month of carnitine supplementation in acetate hemodialysis significantly reduced plasma TG (230 +/- 95 to 136 +/- 20 mg/dl; P less than 0.05) and elevated HDL-C (50 +/- 12 to 71 +/- 26 mg/dl; P less than 0.05). However, this effect was no longer observed at the end of three months of supplementation. Bicarbonate hemodialysis had lower baseline TG values, but carnitine supplementation did not modify plasma lipids (TG:144 +/- 87 to 158 +/- 115 mg/dl; HDL-C:50 +/- 23 to 50 +/- 19 mg/dl). Both groups had a significant increase in plasma carnitine levels after carnitine supplementation. These results suggest that bicarbonate hemodialysis may add a protective effect in hyperlipidemia by reducing requirements of carnitine supplementation. On the other hand, carnitine supplementation should be considered in patients with hyperlipidemia undergoing acetate hemodialysis. The observed difference in response between acetate and bicarbonate hemodialysis may be due to enhanced formation of acetyl-CoA and fatty acid synthesis during acetate hemodialysis.

Acetates↗

The association of facial palsy and/or sensorineural hearing loss in patients with hemifacial microsomia.

Hemifacial microsomia (HFM) is a common craniofacial disorder that is known to be etiologically heterogenous. Phenotypic differentiation of the various subgroups remains unresolved. A review of 50 patients with HFM has yielded data that may help explain different pathogenetic processes. Of particular interest is the association of facial nerve palsy, sensorineural hearing loss (SNHL), or both in a higher percentage of patients than expected. Twenty-two percent had evidence of facial palsy of varying degree. Thirty-three cases had microtia or anotia, and all instances of facial palsy were associated with auricular malformation. Sensorineural hearing loss was found in 16 percent. All patients with microtia and sensorineural hearing loss had facial palsy. Ear tags or pits were found in 21 patients, only two of whom had facial palsy. In all but one case the palsy was found on the more hypoplastic side of the face. In the single exception, both sides of the face were hypoplastic.

Cochlea↗

The laser: an alternative for palliative treatment of obstructing intraluminal lesions.

Twenty nine patients with carcinoma obstructing the colon, six obstructing the gastroesophageal junction, six obstructing the tracheobronchial tree, and one patient with recurrent transitional cell cancer of the bladder invading the rectum, not amenable to surgical resection, were treated by laser photocoagulation. Forty were treated with the Nd:YAG laser and two with the CO2 laser. There were 26 men and 16 women with an average age of 73.8 years. The locations of the tumors were 17 rectal, six rectosigmoid, one transverse colon, two descending colon, four ascending colon, six gastroesophageal, and six in the tracheobronchial tree. Symptoms consisted of obstruction and/or bleeding. Seventeen patients were poor surgical candidates, 25 were unresectable due to widespread disease, two had recurrence at the anastomosis, and three patients had another primary site not controlled by their previous surgical procedure. There were seven complications--three perforations, a rectovaginal fistula, two failures in therapy, and lower GI bleeding. Nineteen patients have died of their disease. Tumors were eradicated in two patients. Forty patients had remained free of referring symptoms of obstruction and bleeding.

Adenocarcinoma↗

Importance of tumor morphology for the long term prognosis of rectal adenocarcinoma.

A retrospective review of 149 consecutive potentially curable rectal adenocarcinomas operated on at our institution between 1965 and 1981 was carried out to assess whether tumor morphology influences long term survival. Complete follow-up was available on all patients and extended to 20 years. Data on tumor morphology was extracted from pathology reports and was available in 147 cases (98.6%). Tumors were defined as exophytic (n = 76) when exhibiting a polypoid growth pattern protruding into the lumen at least 1 cm; when lacking such characteristic growth pattern, tumors were classified under the general term of nonexophytic (n = 71). The 5 year survival rate calculated with life table analysis was 80 per cent for exophytic carcinomas and 54.1 per cent for nonexophytic tumors. This difference was verified by logistic regression analysis and proportional hazard technique and is statistically highly significant (P = 0.001). The rate of distant metastases was 29.7 per cent for exophytic adenocarcinoma and 48.6 per cent for nonexophytic tumors (P = 0.02). Although the results did not reach statistical significance, local recurrence of tumor occurred about twice as often in nonexophytic tumors (15.7%) compared with exophytic tumors (8.1%). Thus, we conclude that tumor morphology is an important preoperative prognostic factor for survival and prediction of distant metastasis, and possibly for local recurrence.

Actuarial Analysis↗

Primary structure of an N-glycosidic carbohydrate unit derived from Sophora japonica lectin.

The lectin isolated from Sophora japonica seeds is a glycoprotein which binds oligosaccharides with non-reducing terminal Gal beta(1----3/4)GlcNac beta 1----units. The carbohydrate moiety of the lectin is composed of fucose, xylose, mannose and N-acetylglucosamine. The major glycopeptide of the lectin, prepared by pronase digestion, was derivatized with fluorescein isothiocyanate, purified by PAGE and examined by exoglycosidase digestion as well as purified by gel filtration through Bio-Gel P6-DG and investigated by methylation analysis and 400-MHz 1H-NMR spectroscopy. The primary structure of the glycopeptide was established to be as follows. (Formula: see text). Structures similar to this containing a (beta 1-2)xylosyl substituent on the core beta-mannosyl residue and an inner core (alpha 1-3)fucosyl substituent seem to occur frequently in plant glycoproteins.

Carbohydrate Conformation↗

Autosomal recessive inheritance in the Setleis bitemporal 'forceps marks' syndrome.

We present the clinical findings in two children with the Setleis bitemporal "forceps marks" syndrome. The striking features include the following: (1) bitemporal scarring, an anomaly that resembles forceps marks; (2) periorbital puffiness with wrinkling of the skin; (3) abnormalities of the eyebrows; (4) anomalies of the eyelashes; (5) flattening of the nasal bridge with a bulbous nasal tip; (6) increased mobility of the skin, associated with severely redundant facial soft tissue; and (7) normal growth and development. The evidence that suggests that this unusual syndrome is inherited in an autosomal recessive fashion includes the following: (1) seven of the patients have come from the relatively isolated towns of San Sebastian and Aguadilla in Puerto Rico; (2) two sets of affected siblings have been described, and, in both cases, the siblings' parents were normal; and (3) one of the children described herein is the product of a consanguineous mating. Although the pathogenetic mechanism is unknown, Setleis syndrome is clearly inherited as an autosomal recessive trait.

Chromosome Aberrations↗

Treatment of retinal detachment with congenital optic pit by krypton laser photocoagulation.

Five patients with a congenital pit of the optic nerve and serous detachment of the macular retina were treated with krypton laser photocoagulation to the juxtapapillary region in an attempt to induce resolution of the retinal detachment. The subretinal fluid resolved after laser therapy in four of the patients. In the fifth, the retinal detachment was successfully treated with pars plana vitrectomy. One patient developed a macular hole and decreased visual acuity, despite resolution of the subretinal fluid.

Adolescent↗

Retinopathy of prematurity. A randomized, prospective trial of transcutaneous oxygen monitoring.

To determine whether the use of continuous transcutaneous oxygen monitoring (tcPO2) could reduce the incidence of retinopathy of prematurity (ROP) in pre-term infants receiving oxygen therapy, a randomized, prospective trial of constant monitoring using the transcutaneous oxygen monitor versus intermittent monitoring of oxygen was performed on a population of premature infants at very high risk for the development of ROP. Two hundred ninety-six infants were randomly assigned to either a constantly monitored (CM) or standard care (SC) group. CM infants had tcPO2 monitored continuously as long as they required supplemental oxygen, whereas SC infants had tcPO2 monitored only during the more acute state of their illness. Management of both groups was otherwise identical. One hundred one of 148 infants in the CM group and 113 of 148 in the SC group survived. The overall incidence of ROP was 51% in the CM group and 59% in the SC group (no significant difference). In infants over 1000 g birthweight, as the weight increased there was increasing risk of developing ROP in the SC group. The odds ratio for the ROPsc:ROPcm reached 7.6 in infants between 1200 and 1300 g in infants with Apgar scores greater than or equal to 8 at 5 minutes. The incidence of cicatricial ROP was similar in both groups: four in the CM and five in the SC group.

Blood Gas Monitoring, Transcutaneous↗

A survey of housestaff attitudes towards terminal care education.

Medical and surgical housestaff have received little formal training to treat the medical or psychosocial problems associated with terminal illness. That more postgraduate terminal care education is needed is generally not debated but what should be taught and how it should be taught are unresolved issues. This paper presents data from a questionnaire given to 173 internal medical and surgical residents intended to provide information on their perceived needs in this area of education. Questions were developed to address four major areas: (1) whether and how terminal care issues should be taught; (2) residents' perceptions of their clinical competence in this area; (3) how personal issues affect care; (4) the residents' roles vis à vis the dying patient and family. A significant portion of the residents were able to acknowledge the importance of their own experience and the need to personally address these issues during training. Thirty-five percent of the sample indicated the need for more training in treating severe pain and 28% felt the need for more training in treating shortness of breath. About 50% of the sample felt that they needed more training in psychosocial areas. This paper presents a method by which training directors can survey their housestaff's perceptions and needs in this area in order to increase the likelihood that terminal care training will match the learning needs and styles of the particular resident group.

Adult↗

Retinopathy of prematurity. Diagnosis, severity, and natural history.

This article presents data gathered in a prospective clinical trial of constant monitoring of oxygen therapy on the diagnosis, severity (as coded by a severity index [SI]) and natural history of retinopathy of prematurity (ROP) in 214 surviving infants with birthweights less than or equal to 1300 g. ROP developed in 119 (55.6%) infants and, of these, cicatricial ROP developed in nine (7.6%). The disease was diagnosed at 8-9 weeks on the average in infants with birthweights less than 900 g and 4-5 weeks in infants 900 to 1300 g. Those infants in whom the disease regressed, ROP lasted approximately 15 weeks. The severity of the disease, as defined by the SI, was strongly correlated with the birthweight of the infant and weakly correlated with the total duration of oxygen therapy.

Cicatrix↗

Excessive lead absorption resulting from exposure to lead naphthenate.

There are no reported studies of the relationship between exposure to lead naphthenate and lead absorption in humans. In an aluminum forging operation where lead naphthenate was sprayed without local ventilation, the mean concentration of lead in air was 96 micrograms/m3 with a range of 12-430 micrograms/m3. The 29 forge operators who worked in this area had a mean blood lead concentration of 63 micrograms/dl, which was statistically significantly (P less than .001) higher than the mean blood lead concentration of 17 micrograms/dl among the 103 unexposed workers. Similarly, the mean zinc protoporphyrin concentration among the forge operators was 265 micrograms/dl, which was statistically significantly (P less than .001) higher than the mean concentration of 26 microgram/dl among the unexposed workers. Nineteen workers, all of whom worked in the forge areas, had blood lead concentrations in excess of 60 micrograms/dl, with the highest concentration being 108 micrograms/dl. This is the first reported instance in which the use of lead naphthenate has been associated with increased lead absorption in humans.

Absorption↗

Floppy eyelid syndrome and blepharochalasis.

Floppy eyelid syndrome and blepharochalasis may represent a spectrum of one underlying disease. Two patients with floppy eyelid syndrome and one with blepharochalasis shared important clinical similarities. All three patients displayed eyelid laxity associated with papillary conjunctivitis and ocular surface abnormalities. Histopathologic findings were similar in the three cases, characterized by chronic conjunctival inflammation with normal tarsal collagen. In all three patients surgical horizontal eyelid shortening led to improvement in symptoms and findings.

Adult↗

Further delineation of Weaver syndrome.

Seven new cases of Weaver syndrome are described, including the first reported case in an adult. Overgrowth is usually but not always present. The combination of characteristic facies and developmental delay, with the peculiar radiographic findings of accelerated dysharmonic osseous maturation and splaying of the distal long bones, is diagnostic of Weaver syndrome.

Adult↗

Chronic acalculous gallbladder disease: multiimaging evaluation with clinical-pathologic correlation.

Despite the recent advances in hepatobiliary imaging, the diagnosis of chronic acalculous gallbladder disease remains difficult. A retrospective study was undertaken to assess the value of a multiimaging approach in detecting chronic acalculous gallbladder disease and in predicting which patients would obtain symptomatic relief after cholecystectomy. Of 199 patients with chronic cholecystitis, 26 (13%) had no gallstones. Of these 26, only 17 (65%) had symptoms related to chronic cholecystitis; in the remainder, the histologic diagnosis was made incidentally. After cholecystectomy, 13 (76%) of the 17 symptomatic patients obtained long-term symptomatic relief, while in four, the symptoms recurred. Among patients with histologic changes of chronic cholecystitis, biliary scintigraphy was the most sensitive technique (sensitivity, 89%). The sensitivity of sonography and oral cholecystography was 61.5% and 66%, respectively. However, for identifying symptomatic patients who may obtain long-term symptomatic relief after cholecystectomy, the accuracy of sonography, oral cholecystography, and biliary scintigraphy was 82%, 86%, and 38%, respectively. When two tests were in agreement the accuracy was 88%. For chronic acalculous cholecystitis, more than one study must be performed in order to make the correct diagnosis and to predict good results from cholecystectomy.

Adult↗